Bilateral massive nephromegaly–A rare presentation of t-cell acute lymphoblastic leukemia

Abstract

Introduction Renal infiltration by leukemia causing massive bilateral nephromegaly is an extremely rare presentation of T-cell acute lymphoblastic leukemia(T-ALL). Case report 18-month-old female toddler presented with fever and progressive abdominal distension of 4–6 weeks duration. Imaging revealed bilateral massively enlarged kidneys with normal excretion. Peripheral blood counts and smear examination was unremarkable and immunophenotypic evaluation of marrow was consistent with T-ALL. Chest imaging was unremarkable. She was started on modified Indian Childhood Collaborative Leukemia Group (ICiCLe) ALL protocol. Even with the best anti-tumor lysis syndrome (TLS) prophylaxis the child required two sessions of hemodialysis. An end-induction morphological remission & end-consolidation negative minimal residual disease (MRD) could be achieved. Conclusion Bilateral massive nephromegaly is an extremely rare presentation of T-ALL. This case emphasizes the unusual presentation, need for prompt remediation of TLS, and most importantly the use of early intensification with four drug anthracycline & dexamethasone-based therapy for the treatment of T-ALL in children.

Bilateral nephromegaly due to direct leukemic cell invasion in the initial and relapse phases of T-cell acute lymphoblastic leukaemia

“…Some pediatric cases at diagnosis showed bilateral nephromegaly without abnormal cells in the circulation. [5] In our case, since the patient had renal dysfunction with abnormal cells in circulation and bicytopenia, it was obvious that he had hematological disease. Bilateral nephromegaly can be caused by various conditions, such as diabetes mellitus, [11] acute glomerulonephritis, [12] acute interstitial nephritis, [13] acute tubular necrosis, [14] rapidly progressive glomerulonephritis, [15] and HIV-associated nephropathy, [16] amyloidosis, [17] malignant or benign infiltrative diseases (multiple myeloma, lymphoma, leukemia, and renal metastasis [18] ), bilateral hydronephrosis, and cystic diseases.…”

“…Renal infiltration of ALL is usually observed in the late stages of the disease. [ 5 ] Only a few pediatric cases have been reported to show bilateral renal infiltration, causing nephromegaly at diagnosis. [ 6 , 7 ] To the best of our knowledge, there are only three reported adult cases of bilateral nephromegaly due to leukemia infiltration at diagnosis.…”

“…Some pediatric cases at diagnosis showed bilateral nephromegaly without abnormal cells in the circulation. [ 5 ]…”

“…The present case showed a rare occurrence of AKI associated with bilateral nephromegaly secondary to T-ALL infiltration into the bilateral tubulointerstitium at the time of T-ALL diagnosis. Renal infiltration of ALL is usually observed in the late stages of the disease [5] . Only a few pediatric cases have been reported to show bilateral renal infiltration, causing nephromegaly at diagnosis [6,7] .…”

“…Interestingly, in these cases, leukemia infiltration into the kidneys was confirmed before bone marrow relapse. Some pediatric cases at diagnosis showed bilateral nephromegaly without abnormal cells in the circulation [5] …”